Endoscopy 2010; 42: E350-E351
DOI: 10.1055/s-0030-1256003
Unusual cases and technical notes

© Georg Thieme Verlag KG Stuttgart · New York

A fulminant course of Cronkhite-Canada syndrome

J.  Martinek1 , T.  Chvatalova1 , F.  Zavada1 , P.  Vankova3 , I.  Tuckova2 , M.  Zavoral1
  • 1Charles University in Prague, Department of Internal Medicine, First Faculty of Medicine and Central Military Hospital, Praha, Czech Republic
  • 2Department of Pathology, Central Military Hospital, Praha, Czech Republic
  • 3Department of Gastroenterology, Jablonec Hospital, Jablonec nad Nisou, Czech Republic
Further Information

J. Martinek, MD, PhD 

Department of Internal Medicine
Central Military Hospital

U Vojenská nemocnice 1200
Praha 6
Czech Republic

Email: jan.martinek@volny.cz

Publication History

Publication Date:
17 December 2010 (online)

Table of Contents

A 57-year-old man suffered from watery diarrhea, weight loss, and abdominal pain for 4 months. Noticeable signs of nail dystrophy (onychomadesis of all nails) were present ([Fig. 1 a]) along with alopecia and cutaneous foci of hyperpigmentation ([Fig. 1 b]).

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Fig. 1 Main physical findings. a All 20 nails with onychomadesis about 3 mm from the lunula. Several nail plates have been lost. b Hands with several small foci of skin hyperpigmentation.

Upper-gastrointestinal endoscopy revealed a large number of strawberry-like polyps of different size in the stomach ([Fig. 2]).

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Fig. 2 Endoscopic findings in the stomach. a Polyps of gastric body and antrum. b Detailed view of gastric polyp using zoom and narrow-band imaging mode; note the strawberry-like appearance.

Colonoscopy revealed polyposis of the whole colon, including the rectum. The majority of the polyps had a strawberry-like, adenomatous, and hyperplastic appearance ([Fig. 3]).

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Fig. 3 a, b Endoscopic view of colonic polyps.

Histologically, the majority of the polyps were juvenile-like with cystic dilatations of the glands and a benign mucinous epithelium. The glands were filled with a large amount of mucin ([Fig. 4]).

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Fig. 4 Histology. a Juvenile-like polyp with enlarged mucin glands. b Detailed view of mucinous glandular epithelium.


Quality:

Video 1 Endoscopy of the stomach.


Quality:

Video 2 Endoscopy of the colon.

Some adenomatous polyps with low-grade dysplasia in the colon were also detected. A subsequent enteroscopy did not find polyps; however, edema and small indentation of the jejunum were present. Immunohistochemistry showed total alactasia and a strong positivity for tumor necrosis factor (TNF) in the macrophages and lymphocytes. Based on both the clinical and endoscopic picture, a diagnosis of Cronkhite-Canada syndrome was established.

Despite complex treatment, the clinical course was unfavourable. Since the clinical state of the patient did not improve, anti-TNF-α treatment was considered. TNF-α activity was examined in the small-intestinal mucosa, and the results showed a strong intracellular expression of TNF-α. Unfortunately, an experimental anti-TNF-α treatment could not be introduced, because of the rapid progression of the disease. The patient died 4 months after the diagnosis of Cronkhite-Canada syndrome was established.

Cronkhite-Canada syndrome is a rare non-hereditary polyposis syndrome of an unknown, possibly autoimmune etiology. The prognosis is poor and more than 50 % of patients die within 2 – 4 years [1] [2]. Cronkhite-Canada syndrome should be considered in all patients with gastrointestinal polyposis together with emergent alopecia and/or nail dystrophy [3].

Competing interests: None

Endoscopy_UCTN_Code_CCL_1AD_2AC

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References

J. Martinek, MD, PhD 

Department of Internal Medicine
Central Military Hospital

U Vojenská nemocnice 1200
Praha 6
Czech Republic

Email: jan.martinek@volny.cz

#

References

J. Martinek, MD, PhD 

Department of Internal Medicine
Central Military Hospital

U Vojenská nemocnice 1200
Praha 6
Czech Republic

Email: jan.martinek@volny.cz

Zoom Image

Fig. 1 Main physical findings. a All 20 nails with onychomadesis about 3 mm from the lunula. Several nail plates have been lost. b Hands with several small foci of skin hyperpigmentation.

Zoom Image

Fig. 2 Endoscopic findings in the stomach. a Polyps of gastric body and antrum. b Detailed view of gastric polyp using zoom and narrow-band imaging mode; note the strawberry-like appearance.

Zoom Image

Fig. 3 a, b Endoscopic view of colonic polyps.

Zoom Image

Fig. 4 Histology. a Juvenile-like polyp with enlarged mucin glands. b Detailed view of mucinous glandular epithelium.